We Can Adjust The Sails

June has been a mad month.  I’ve been frantically trying to juggle the balls of life.  With the slightest hint of summer rays and improved health, I’ve been filling my diary with activities and making memories.  However the lovely NHS has also been filling my diary with weekly hospital visits and doctors appointments.  For the past month, my routine has been a full day activity (fun or hospital) followed by two days recovery… then a full days activity followed by two days recovery.  It has been exhausting, and I’ve begun to look at my calendar with dread, desperately searching for the end of the manic-ness.  Therefore when an appointment came through for me to return to the Brompton to see Professor Wells about my steroids, I wasn’t too impressed!  I’d only seen his colleague a few weeks ago, so tried desperately to cancel or postpone it, but without luck.  However it turned out to be a really interested meeting.

Cuddles with Lottie!

Lottie!

A dog walk!

Phil was pretty hospital-ed out with all of our visits recently, so didn’t want to take time off again for what we thought would be a pointless meeting.  My Dad normally takes me to any appointments in Oxfordshire that are tricky for Phil, however the journey to London is too far for him from his home town.  Therefore for the first time ever I was stuck how to get to the Brompton, as I’m not well enough to drive or use public transport.  However, the wonderful NHS came to the rescue again, they can provide ambulance cars for this exact situation.  The criteria to be eligible is quite strict, essentially I passed as I use oxygen and have reduced mobility.  They originally wanted to send me in a full on ambulance, with two crew members!  That seemed like a ridiculous waste of resources, so they agreed to let me go in an ambulance car if I brought my own oxygen from home and was happy to administer it myself!  My driver was a lovely retired gentleman, who volunteers every day to drive people around the county on behalf of the ambulance service.  What a kind guy.  After dropping me off, he then waited in the ambulance area, chatting to the other crews whilst I was in my appointment, before driving me home.  It was brilliant, as I was so exhausted and feeling very poorly, but could completely relax knowing there was someone looking after me, who would get me home even if I was ill.  It is a valuable service, another demonstration of how many cogs make the NHS work.  And another example of why we have an amazing health service.

I was pretty exhausted and breathless before I’d even got there.  I’d spent the evening before the appointment waving my hands along to Elton John, and had gone to bed pretty late.  Therefore I was really not happy with my first task: do the six minute walk test again.. but without oxygen!!  I’d been dreading this, knowing how ill walking used to make me feel before I started using oxygen.  In fact I had disputed the reasoning of this a fair few times with the nurse in the lead up to the appointment!  But it turns out the doctor knows best, and there was a reason for making me do this test!  As amazingly, despite feeling poorly, I managed 190m!  And amazingly my oxygen saturations only fell to 90%!  Gobsmacked!

The ‘pointless meeting’ with Professor Wells, turned out to be incredibly interesting!  Instead of a quick meeting, it turned in to two long joint discussions with both him and his respiratory colleague, and my two PH doctors.  It turns out that I am incredibly lucky, as Professor Wells is a world leader in auto immune diseases of the lungs… and apparently that is what I have as well!  I was convinced that the steroids had not helped my lungs.  However, back in February, prior to going on them, my oxygen saturations were falling to the 80s walking across the room, and the 70s walking up stairs.  Back in February I would not have been able to walk for six minutes without oxygen.  However amazingly, even though I was feeling poorly, my lungs were able to maintain my sats at a reasonable level for a short time if I walked slowly.  Professor Wells believes I am having an auto-immune reaction to the PVOD in my lungs, this is causing extra heat around the blood vessels which is making it even harder for me to breathe.  Therefore by giving me steroids, it has helped reduce the immune reaction, and so helped improve my breathing slightly.  So incredibly the steroids have improved the lungs functioning a little- a complete surprise as I wasn’t expecting anything to be able to help them.  However this does mean that I now have a third condition to add to my list!  I remember back in March, they were narrowing down the conditions, and were deciding between PVOD and an auto-immune problem- now it appears I have both of those problems.. getting ill in style!!  At the end of the meeting, for the first time ever, all of the doctors were convinced that they have now solved it all.  They think they can explain all of my problems and symptoms for the past three years with those three conditions: Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease and an Auto-Immune Disregulation.  For the first time ever, they think they have got to the bottom of everything.  Relief.  Answers stop you feeling like you’re going mad, making up symptoms, being lazy for not getting better.  Answers stop you worrying, stop you hypothesising, stop you stressing about what they’re missing.  Relief.

So it seems like steroids and I are going to be together forever.  Since having my dosage reduced a month ago, I have noticed a great reduction in side effects, to a level where it is now manageable.  My moonface has not improved though, so I think we’re all going to have to get used to me and my rounded face!  I am going to have my steroids reduced again to 10 mg daily, but I will start taking an immune-suppressant (mycophenolate) as well.  Apparently the combination of those two drugs at low dosages, is as good as just one of them at a higher dose.  And by being on a low dose, it will help reduce side effects further.  I’m a little concerned that I’m going to be even more immuno-suppressed from now on, so i’ll have to avoid ill people as they can make me really poorly!

After the meeting I sat in the car on the way home and processed all of what the doctors had been saying to me.  At some point in the last few months, conversations have changed, wording has been revised, goals have been altered.  The changes have been subtle, and it was only then as I sat in the car that I realised about it.  Now the conversations are about giving me extra time, keeping me alive for transplant, helping my body to remain strong enough to cope.  Essentially no longer are we discussing medicines to enjoy life and to keep me around for many years to come, it’s now all about how to prolong me for a little bit longer.  A small subtle change, but one that I realised in that car on the way home.  They are indirectly reminding me that I am nearing the end of my path.  I just hope that another path (transplant) is going to be added at some point.

However as I sat in the car, I also felt pleasantly surprised and relieved that my lungs’ ability to breathe and maintain saturation has improved a little.  I knew that I’d been feeling better, but had completely dismissed the idea that the steroids might be helping.  I am very thankful that the respiratory doctors got involved in my case, else I dread to think how ill I would be now.  Someone is looking out for me at the moment.  I am also very relieved that we apparently seem to have solved everything (hopefully).  It has taken three and a half years, it has taken tens of doctors, it has taken hundreds of tests, it has taken thousands of pounds… but the wonderful NHS has apparently solved the problem of me and my illnesses.  An amazing service, I only hope that we continue to invest in it and look after it.  So those that come after me, can have their problems solved too.